Erdheim-Chester disease is a rare non-langerhans systemic histiocytosis of unknown origin, associated with bone diseases and severe visceral complications. Therapies have been disappointing. A recombinant form of interleukin-1 receptor antagonist (anakinra) has been used in a few cases when usual treatment fails. We report a new case of successfully interleukin-1 receptor antagonist treatment in Erdheim-Chester disease.
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